Analysis of lasp function in myofibril assembly

我们建议研究LASP蛋白，这是正常肌肉功能所必需的。肌肉最小的收缩单位有两个细丝系统，粗丝和细丝相互滑动以产生收缩力。LASP的突变会导致较短的细丝，因此会损害肌肉功能，因为较短的细丝可以产生的收缩力较小。我们将研究LASP如何通过遗传、生化和先进的成像方法来调节细丝长度，例如细丝周转的实时成像。这些基础细胞生物学研究将阐明肌肉功能在发育和疾病中的重要方面。

We propose to investigate the protein Lasp, which is essential for proper muscle function. The smallest contractile units of muscles have two filament systems, the thick and thin filaments that slide past each other to generate contractile force. A mutation in Lasp results in shorter thin filaments, and therefore impaired muscle function, as the contractile force that can be generated with shorter thin filaments is smaller. We will investigate how Lasp regulates thin filament length with genetic, biochemical, and advanced imaging methods, such as live imaging of thin filament turnover. These basic cell biological studies will shed light on important aspects of muscle function in development and disease.